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The Clinical and Pathological Features of IgG4-Related Disease

  • VASCULITIS (LUIS R. ESPINOZA, SECTION EDITOR)
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Abstract

The rapidly emerging disorder now known as IgG4-related disease (IgG4-RD) includes a variety of clinical entities once regarded as being entirely separate diseases. Manifestations of IgG4-RD have now been reported in essentially all organ systems. Regardless of which organ is involved, tissue biopsies reveal striking histopathological similarities. The hallmark pathology findings are diffuse lymphoplasmacytic infiltrates, abundant IgG4-positive plasma cells, modest tissue eosinophilia, and extensive fibrosis. Tumorous swelling and obliterative phlebitis are other frequently observed features. Polyclonal elevations of serum IgG4 are found in approximately 70% of patients. Many questions pertaining to the etiology, pathophysiology, epidemiology, clinical features, therapy, disease monitoring, and long-term outcomes remain to be addressed. This paper focuses on the clinical and pathological features of IgG4-RD.

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Authors and Affiliations

  1. Rheumatology Unit (Department of Medicine), Massachusetts General Hospital, Harvard Medical School, Boston, MA, 02114, USA

    Arezou Khosroshahi & John H. Stone

  2. Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, 02114, USA

    Vikram Deshpande

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  1. Arezou Khosroshahi
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Khosroshahi, A., Deshpande, V. & Stone, J.H. The Clinical and Pathological Features of IgG4-Related Disease. Curr Rheumatol Rep 13, 473–481 (2011). https://doi.org/10.1007/s11926-011-0213-7

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